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Coagulation and fibrinolytic features in AL amyloidosis with abnormal bleeding and usefulness of tranexamic acid

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Abstract

Abnormal bleeding is sometimes observed in patients with immunoglobulin light chain (AL) amyloidosis. Although several theories have been proposed regarding the pathological causes of the bleeding tendency in AL amyloidosis, many lacked sufficient evidence and full consensus. We conducted a retrospective survey at a single institution to assess bleeding manifestations, methods for evaluating hematological abnormalities, and treatments for bleeding in patients with systemic AL amyloidosis over the past 13 years. The participants were 10 men and 14 women, aged 39–84 years (mean 65 years). The prevalence of bleeding was 29%. Prolonged prothrombin time (PT), elevated plasmin–α2-antiplasmin complex, and factor X deficiency were distinctive to the bleeding group. Two case studies showed that tranexamic acid was effective for treating this hematological condition. However, two patients with normal PT and activated partial thromboplastin time (APTT) also had a bleeding manifestation. The rates of administration of coagulation and fibrinolytic tests were relatively low in the non-bleeding group. Therefore, a close investigation concerning coagulation and fibrinolysis should be performed in every patient with AL amyloidosis regardless of the PT/APTT values. A more careful, comprehensive, and large-scale study is required to reinforce these findings.

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Acknowledgements

This study was supported in part by a grant-in-aid from the Japanese Ministry of Health, Labour, and Welfare (to A.I.).

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Correspondence to Masahisa Arahata.

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H. Takamatsu reports grants and personal fees from Celgene, Bristol–Myers Squibb and Ono pharmaceutical, grants from CSL Behring and SRL, and personal fees from Bristol–Myers Squibb, Janssen Pharmaceutical, Sanofi, Takeda Pharmaceutical, Fujimoto Pharmaceutical, Becton, Dickinson and Company, outside the submitted work. The other authors report no conflict of interest.

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Arahata, M., Takamatsu, H., Morishita, E. et al. Coagulation and fibrinolytic features in AL amyloidosis with abnormal bleeding and usefulness of tranexamic acid. Int J Hematol 111, 550–558 (2020). https://doi.org/10.1007/s12185-019-02811-x

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  • DOI: https://doi.org/10.1007/s12185-019-02811-x

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